Abstract
Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory disease from the group of multifactorial diseases. It is characterized by non-infectious recurrent bone lesions, usually manifesting in childhood. The aim of this study was to analyze the clinical, laboratory, radiological and histological data of 33 patients (12 boys, 21 girls) with a diagnosis of chronic nonbacterial osteomyelitis. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. 25/33 patients were hospitalized to the Center with suspected neoplastic disease bone. The median age of CNO manifestation was 9.6 years. 24/33 had a multi-focal form of the disease. 5 patients had fever. Nonsteroidal anti-inflammatory drugs were the first therapy for all patients, achieving clinical remission in 4 cases. Zoledronic acid were used in 29 patients with remission in 19, adalimumab in 10 patient with remission in 9. In one patient stabilization was achieved only with the combination of methotrexate and canakinumab therapy.
Highlights
ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им
Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory disease from the group of multifactorial diseases. It is characterized by non-infectious recurrent bone lesions, usually manifesting in childhood
Nonsteroidal anti-inflammatory drugs were the first therapy for all patients, achieving clinical remission in 4 cases
Summary
Характеризуется неинфекционным рецидивирующим поражением костной ткани, как правило, манифестирующим в детском возрасте. Дмитрия Рогачева были госпитализированы 25/33 пациентов с подозрением на течение неопластического процесса костной ткани. Десять из 29 пациентов в последующем получили антицитокиновую терапию (адалимумаб), ремиссия была достигнута в 9 случаях. Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory disease from the group of multifactorial diseases. It is characterized by non-infectious recurrent bone lesions, usually manifesting in childhood. Хронический небактериальный остеомиелит (ХНО) – это разнородная группа аутовоспалительных заболеваний, характеризующихся хроническим неинфекционным воспалительным поражением костной ткани с единичным или множественными очагами поражения, склонное к рецидивированию [1,2,3,4,5]. Синдром Маджид, вызванный мутациями в гене LPIN2 [30] и синдром DIRA, вызванный мутациями в гене IL1RN, сопровождаются избыточным синтезом провоспалительных цитокинов, в первую очередь ИЛ-1, и отвечают на терапию антагонистами ИЛ-1 [7, 31, 32]
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